Ineffective esophageal motility: Characterization and outcomes across pediatric neurogastroenterology and motility centers in the United States.

OBJECTIVES: Ineffective esophageal motility (IEM) on high-resolution manometry (HRM) is not consistently associated with specific clinical syndromes or outcomes. We evaluated the prevalence, clinical features, management, and outcomes of pediatric IEM patients across the United States. METHODS: Clinical and manometric characteristics of children undergoing esophageal HRM during 2021-2022 were collected from 12 pediatric motility centers. Clinical presentation, test results, management strategies, and outcomes were compared between children with IEM and normal HRM. RESULTS: Of 236 children (median age 15 years, 63.6% female, 79.2% Caucasian), 62 (23.6%) patients had IEM, and 174 (73.7%) patients had normal HRM, with similar demographics, medical history, clinical presentation, and median symptom duration. Reflux monitoring was performed more often for IEM patients (25.8% vs. 8.6%, p = 0.002), but other adjunctive testing was similar. Among 101 patients with follow-up, symptomatic cohorts declined in both groups in relation to the initial presentation (p > 0.107 for each comparison) with management targeting symptoms, particularly acid suppression. Though prokinetics were used more often and behavioral therapy less often in IEM (p ≤ 0.015 for each comparison), symptom outcomes were similar between IEM and normal HRM. Despite a higher proportion with residual dysphagia on follow-up in IEM (64.0% vs. 39.1%, p = 0.043), an alternate mechanism for dysphagia was identified more often in IEM (68.8%) compared to normal HRM (27.8%, p = 0.017). CONCLUSIONS: IEM is a descriptive manometric pattern rather than a clinical diagnosis requiring specific intervention in children. Management based on clinical presentation provides consistent symptom outcomes.

Interrater Reliability of Functional Lumen Imaging Probe Panometry and High-Resolution Manometry for the Assessment of Esophageal Motility Disorders.

INTRODUCTION: High-resolution manometry (HRM) and functional lumen imaging probe (FLIP) are primary and/or complementary diagnostic tools for the evaluation of esophageal motility. We aimed to assess the interrater agreement and accuracy of HRM and FLIP interpretations. METHODS: Esophageal motility specialists from multiple institutions completed the interpretation of 40 consecutive HRM and 40 FLIP studies. Interrater agreement was assessed using intraclass correlation coefficient (ICC) for continuous variables and Fleiss' κ statistics for nominal variables. Accuracies of rater interpretation were assessed using the consensus of 3 experienced raters as the reference standard. RESULTS: Fifteen raters completed the HRM and FLIP studies. An excellent interrater agreement was seen in supine median integral relaxation pressure (ICC 0.96, 95% confidence interval 0.95-0.98), and a good agreement was seen with the assessment of esophagogastric junction (EGJ) outflow, peristalsis, and assignment of a Chicago Classification version 4.0 diagnosis using HRM (κ = 0.71, 0.75, and 0.70, respectively). An excellent interrater agreement for EGJ distensibility index and maximum diameter (0.91 [0.90-0.94], 0.92 [0.89-0.95]) was seen, and a moderate-to-good agreement was seen in the assignment of EGJ opening classification, contractile response pattern, and motility classification (κ = 0.68, 0.56, and 0.59, respectively) on FLIP. Rater accuracy for Chicago Classification version 4.0 diagnosis on HRM was 82% (95% confidence interval 78%-84%) and for motility diagnosis on FLIP Panometry was 78% (95% confidence interval 72%-81%). DISCUSSION: Our study demonstrates high levels of interrater agreement and accuracy in the interpretation of HRM and FLIP metrics and moderate-to-high levels for motility classification in FLIP, supporting the use of these approaches for primary or complementary evaluation of esophageal motility disorders.

The Utility of Functional Luminal Imaging Probes Measurements to Diagnose Dysmotility and Their Relationship to Impaired Bolus Clearance.

BACKGROUND: Functional luminal imaging probes (FLIP) have been used by multiple centers to assess esophagogastric junction (EGJ) function in patients at risk for esophageal obstruction but its role in diagnosing peristaltic disorders is less well studied. In particular, there are no studies comparing the sensitivity of FLIP to diagnose motility abnormalities and impaired bolus transit by high-resolution esophageal manometry with impedance. METHODS: We prospectively recruited 42 patients undergoing high-resolution esophageal manometry with impedance (HRIM) who also underwent FLIP between 2018 and 2020. HRIM parameters were analyzed using Swallow Gateway software to determine peristaltic and lower esophageal sphincter pressure measurements as well as bolus flow parameters. FLIP tracings were analyzed for the presence of repetitive antegrade contractions (RACs), EGJ distensibility, and associated parameters. RESULTS: Forty-two patients were included (11 controls, 7 achalasia, 16 fundoplication, 8 dysmotility). The mean age of patients was 10.1 ±â€Š0.9 years. There were significant differences in bolus flow parameters across diagnosis with longer bolus presence (BPT) in control patients compared with fundoplication and dysmotility patients. There was a significant correlation between EGJ diameter, EGJ distensibility and bolus flow time (BFT) for solid foods (r2 > 0.518, P < 0.02). The presence of RACs and EGJ relaxation during RACs was associated with a greater BFT and BPT across textures (P < 0.05). Forty-two percentage of patients with absent RACs, however, had clear peristalsis by HRIM. CONCLUSIONS: The presence of RACs and EGJ relaxation by FLIP correlate with improved bolus flow. Patients with an absence of RACs need HRIM to confirm any diagnoses of dysmotility.

Classifying Esophageal Motility by FLIP Panometry: A Study of 722 Subjects With Manometry.

INTRODUCTION: Functional luminal imaging probe (FLIP) panometry can evaluate esophageal motility in response to sustained esophageal distension at the time of sedated endoscopy. This study aimed to describe a classification of esophageal motility using FLIP panometry and evaluate it against high-resolution manometry (HRM) and Chicago Classification v4.0 (CCv4.0). METHODS: Five hundred thirty-nine adult patients who completed FLIP and HRM with a conclusive CCv4.0 diagnosis were included in the primary analysis. Thirty-five asymptomatic volunteers ("controls") and 148 patients with an inconclusive CCv4.0 diagnosis or systemic sclerosis were also described. Esophagogastric junction (EGJ) opening and the contractile response (CR) to distension (i.e., secondary peristalsis) were evaluated with a 16-cm FLIP during sedated endoscopy and analyzed using a customized software program. HRM was classified according to CCv4.0. RESULTS: In the primary analysis, 156 patients (29%) had normal motility on FLIP panometry, defined by normal EGJ opening and a normal or borderline CR; 95% of these patients had normal motility or ineffective esophageal motility on HRM. Two hundred two patients (37%) had obstruction with weak CR, defined as reduced EGJ opening and absent CR or impaired/disordered CR, on FLIP panometry; 92% of these patients had a disorder of EGJ outflow per CCv4.0. DISCUSSION: Classifying esophageal motility in response to sustained distension with FLIP panometry parallels the swallow-associated motility evaluation provided with HRM and CCv4.0. Thus, FLIP panometry serves as a well-tolerated method that can complement, or in some cases be an alternative to HRM, for evaluating esophageal motility disorders.

Comparison of Aerodigestive and Nonaerodigestive Provider Responses to Clinical Case Vignettes.

OBJECTIVE: To evaluate differences in practice patterns between aerodigestive and nonaerodigestive providers in pediatric gastroenterology when diagnosing and treating common aerodigestive complaints. STUDY DESIGN: A questionnaire comprised of clinical vignettes with multiple-choice questions was distributed to both aerodigestive and nonaerodigestive pediatric gastroenterologists. Vignettes focused on management of commonly encountered general gastroenterology and aerodigestive issues, such as gastroesophageal (GE) reflux, aspiration, and feeding difficulties. Tests of equal proportions were used to compare rates of testing and empiric therapy within and across groups. Multivariate analysis was used to assess differences in response rates between aerodigestive and nonaerodigestive providers. RESULTS: A total of 88 pediatric gastroenterologists from 18 institutions completed the questionnaire. There were 35 aerodigestive gastroenterology providers and 53 nonaerodigestive gastroenterology providers. The nonaerodigestive group included 31 general gastroenterologists and 22 providers with self-identified subspecialty gastroenterology expertise. Aerodigestive specialists were more likely than nonaerodigestive gastroenterologists to pursue testing over empiric therapy in cases involving isolated respiratory symptoms (P < .05); aerodigestive providers were more likely to recommend pH-impedance testing, videofluoroscopic swallow studies, and upper gastrointestinal barium study (P < .05 for each test) depending on the referring physician. For vignettes involving infant GE reflux, both groups chose empiric treatments more frequently than testing (P < .001), although aerodigestive providers were more likely than nonaerodigestive providers to pursue testing like upper gastrointestinal barium studies (P < .05). CONCLUSIONS: Although some practice patterns were similar between groups, aerodigestive providers pursued more testing than nonaerodigestive providers in several clinical scenarios including infants with respiratory symptoms and GE reflux.

A Collaborative Effort to Advance Drug Development in Pediatric Constipation and Irritable Bowel Syndrome.

ABSTRACT: Pediatric functional gastrointestinal disorders including irritable bowel syndrome with constipation and functional constipation are common conditions in childhood, but no drugs are U.S. Food and Drug Administration (FDA) approved for chronic use in pediatric patients with these disorders. Despite efforts to better standardize the diagnosis of these conditions in children (including recent modifications to the Rome criteria), conducting pediatric clinical trials to support drug approval remains a challenge. In March 2018, FDA, in collaboration with the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition, American Gastroenterological Association, and American College of Gastroenterology, convened a public workshop to discuss the challenges and opportunities in conducting pediatric clinical trials in functional gastrointestinal conditions. The workshop assembled gastroenterologists, psychologists, patients, patient advocates, regulators, and industry representatives to discuss trial design and conduct including alternative designs, eligibility criteria, instruments for patient- and observer-reported outcomes, and optimal primary endpoints to support regulatory approval. This report summarizes the workshop, key challenges and knowledge gaps identified, and outlines areas where further research efforts are needed to overcome barriers to developing drugs to treat these conditions.

American Neurogastroenterology and Motility Society Task Force Recommendations for Resumption of Motility Laboratory Operations During the COVID-19 Pandemic.

The American Neurogastroenterology and Motility Society Task Force recommends that gastrointestinal motility procedures should be performed in motility laboratories adhering to the strict recommendations and personal protective equipment (PPE) measures to protect patients, ancillary staff, and motility allied health professionals. When available and within constraints of institutional guidelines, it is preferable for patients scheduled for motility procedures to complete a coronavirus disease 2019 (COVID-19) test within 48 hours before their procedure, similar to the recommendations before endoscopy made by gastroenterology societies. COVID-19 test results must be documented before performing procedures. If procedures are to be performed without a COVID-19 test, full PPE use is recommended, along with all social distancing and infection control measures. Because patients with suspected motility disorders may require multiple procedures, sequential scheduling of procedures should be considered to minimize need for repeat COVID-19 testing. The strategies for and timing of procedure(s) should be adapted, taking into consideration local institutional standards, with the provision for screening without testing in low prevalence areas. If tested positive for COVID-19, subsequent negative testing may be required before scheduling a motility procedure (timing is variable). Specific recommendations for each motility procedure including triaging, indications, PPE use, and alternatives to motility procedures are detailed in the document. These recommendations may evolve as understanding of virus transmission and prevalence of COVID-19 infection in the community changes over the upcoming months.

Functional Luminal Imaging Probe Assessment in Postfundoplication Patients Changes Management Beyond Manometry.

OBJECTIVES: Fundoplication is a commonly performed pediatric surgery but the surgery can result in nonspecific symptoms that merit further evaluation. The goal of this study was to determine the utility of Functional Luminal Imaging Probe (FLIP) compared with high-resolution manometry with impedance (HRIM) in the evaluation of fundoplication symptoms. METHODS: We reviewed the FLIP and HRIM tracings of symptomatic fundoplication patients. We compared FLIP results of fundoplication patients to those of pediatric achalasia and control patients. We also compared the role of FLIP to HRIM in clinical decision-making and clinical outcomes. RESULTS: EGJ distensibilities of fundoplication patients ranged from 0.6 to 8.2 mm/mmHg. Because of the different pediatric patient sizes (8.9--73.5 kg), balloon size inflations varied but, after adjusting balloon fill volume by weight, there was a linear relationship between inflation and distensibility. When compared with control patients and achalasia patients, distensibilities of fundoplication patients were lower than control patients and higher than achalasia patients (P = 0.0001). Patients who had an EGJ intervention had a mean EGJ distensibility of 2.3 ±â€Š1.1 mm/mmHg compared with 5.1 ±â€Š1.6 mm/mmHg in medically managed patients (P = 0.0001). Patients who had an esophagogastric junction (EGJ) intervention had a mean IRP of 13.9 ±â€Š6.1 mmHg compared with medically managed patients who had a mean IRP of 9.9 ±â€Š3.9 mmHg (P = 0.3). CONCLUSIONS: We show FLIP may provide additional insight into EGJ physiology in symptomatic fundoplication patients and complements HRIM.

Proceedings of the 2018 Advances In Motility and In NeuroGastroenterology: AIMING for the Future Single Topic Symposium.

OBJECTIVES: Motility and functional disorders are common in children and often debilitating, yet these disorders remain challenging to treat effectively. At the 2018 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the Neurogastroenterology and Motility Committee held a full day symposium entitled, 2018 Advances In Motility and In NeuroGastroenterology - AIMING for the future. The symposium aimed to explore clinical paradigms in pediatric gastrointestinal motility disorders and provided a foundation for advancing new scientific and therapeutic research strategies. METHODS: The symposium brought together leading experts throughout North America to review the state of the art in the diagnosis and management of motility and functional disorders in children. Presentations were divided into esophageal, antral duodenal, and colorectal modules. Each module included oral presentations by experts in the respective fields, leading to thought-provoking discussions. There were 2 breakout sessions with small group discussions on select topics, focusing on defining scientific insights into the diagnosis and management of pediatric functional gastrointestinal and motility disorders in a systematic, segment-based approach. CONCLUSIONS: The field of neurogastroenterology has made remarkable progress in the last decade. The current report summarizes the major learning points from the symposium highlighting the diagnosis and promising therapies on the horizon for pediatric neurogastrointestinal and motility disorders.

Pediatric Eosinophilic Esophagitis Symptom Scores (PEESS v2.0) identify histologic and molecular correlates of the key clinical features of disease.

BACKGROUND: The Pediatric Eosinophilic Esophagitis Symptom Score (PEESS v2.0) measures patient-relevant outcomes. However, whether patient-identified domains (dysphagia, gastroesophageal reflux disease [GERD], nausea/vomiting, and pain) align with clinical symptomology and histopathologic and molecular features of eosinophilic esophagitis (EoE) is unclear. OBJECTIVE: The purpose of this study was to determine whether clinical features of EoE, measured through PEESS v2.0, associate with histopathologic and molecular features of EoE. This represents a novel approach for analysis of allergic diseases, given the availability of allergic tissue biopsy specimens. METHODS: We systematically recruited treated and untreated pediatric patients with EoE (aged 2-18 years) and examined parent proxy-reported symptoms using the PEESS v2.0. Clinical symptomology was collected by questionnaire. Esophageal biopsy samples were quantified for levels of eosinophils, eosinophil peroxidase (EPX) immunohistochemical staining, and mast cells. Molecular features were assessed by using the EoE Diagnostic Panel (94 EoE-related gene transcripts). Associations between domain scores and clinical symptoms and biological features were analyzed with Wilcoxon rank sum and Spearman correlation. RESULTS: The PEESS v2.0 domains correlated to specific parent-reported symptoms: dysphagia (P = .0012), GERD (P = .0001), and nausea/vomiting (P < .0001). Pain correlated with multiple symptoms (P < .0005). Dysphagia correlated most strongly with overall histopathology, particularly in the proximal esophagus (P ≤ .0049). Markers of esophageal activity (EPX) were significantly associated with dysphagia (strongest r = 0.37, P = .02). Eosinophil levels were more associated with pain (r = 0.27, P = .06) than dysphagia (r = 0.24, P = .13). The dysphagia domain correlated most with esophageal gene transcript levels, predominantly with mast cell-specific genes. CONCLUSION: We have (1) established a validated, parent proxy-reported measure for pediatric EoE, the PEESS v2.0; (2) verified that the parent proxy effectively captures symptoms; (3) determined that the dysphagia domain most closely aligns with symptoms and tissue-based molecular biomarkers; (4) established that symptoms correlate with EPX staining; and (5) observed association between mast cells and dysphagia.

Molecular diagnosis of eosinophilic esophagitis by gene expression profiling.

BACKGROUND & AIMS: Gene expression profiling provides an opportunity for definitive diagnosis but has not yet been well applied to inflammatory diseases. Here we describe an approach for diagnosis of an emerging form of esophagitis, eosinophilic esophagitis (EoE), which is currently diagnosed by histology and clinical symptoms. METHODS: We developed an EoE diagnostic panel (EDP) comprising a 96-gene quantitative polymerase chain reaction array and an associated dual-algorithm that uses cluster analysis and dimensionality reduction using a cohort of randomly selected esophageal biopsy samples from pediatric patients with EoE (n = 15) or without EoE (non-EoE controls, n = 14) and subsequently vetted the EDP using a separate cohort of 194 pediatric and adult patient samples derived from both fresh or formalin-fixed, paraffin-embedded tissue: active EoE (n = 91), control (non-EoE and EoE remission, n = 57), histologically ambiguous (n = 34), and reflux (n = 12) samples. RESULTS: The EDP identified adult and pediatric patients with EoE with approximately 96% sensitivity and approximately 98% specificity, and distinguished patients with EoE in remission from controls, as well as identified patients exposed to swallowed glucorticoids. The EDP could be used with formalin-fixed, paraffin-embedded tissue RNA and distinguished patients with EoE from those with reflux esophagitis, identified by pH-impedance testing. Preliminary evidence showed that the EDP could identify patients likely to have disease relapse after treatment. CONCLUSIONS: We developed a molecular diagnostic test (referred to as the EDP) that identifies patients with esophagitis in a fast, objective, and mechanistic manner, offering an opportunity to improve diagnosis and treatment, and a platform approach for other inflammatory diseases.

Impedance probe testing prior to pediatric airway reconstruction.

OBJECTIVE: This study aimed to determine if preoperative impedance testing changed management and if testing was associated with surgical outcome in patients undergoing airway reconstruction. METHODS: Retrospective review of patients who had impedance testing prior to airway reconstruction at a tertiary pediatric hospital from January 2010 to September 2011. Charts were reviewed for demographics, medical/surgical history, impedance testing, and surgical outcomes. RESULTS: Fifty-seven patients were included. Forty-seven (82%) were premature. Forty-seven (82%) had a primary diagnosis of subglottic stenosis. Twenty-six (45%) had prior airway surgery. Thirty-six (63%) had gastroesophageal reflux and 21 (36%) had undergone fundoplication. Patients without fundoplication had a median 46 total reflux, 7 proximal, and 14.5 acidic events compared to a median 5 total reflux, 0 proximal, and 0 acidic events in patients with fundoplication. Impedance testing changed management in 22% (8/36) of nonfundoplication patients and 9.5% (2/21) of fundoplication patients. In unadjusted analysis, fewer fundoplication patients had successful surgery compared to those without (33% vs 67%, P = .01). Prematurity, age at surgery, and previous airway surgery were also important predictors of surgical success. CONCLUSION: Fewer patients than anticipated had a change in management. Impedance testing was unlikely to change management in fundoplication patients. Patients with fundoplication were less likely to have a successful outcome, suggesting that factors other than reflux influence airway reconstruction outcomes.

Venous thrombotic events in hospitalized children and adolescents with inflammatory bowel disease.

BACKGROUND: Adults with inflammatory bowel disease (IBD) have an increased risk of venous thrombotic events (TEs). We sought to evaluate the risk for TE in children and adolescents with IBD using a large population database. METHODS: The triennial Healthcare Cost and Utilization Project Kids' Inpatient Database was used in a retrospective cohort study of hospitalized children in the United States across 1997, 2000, 2003, 2006, and 2009. Billing codes were used to identify discharges with Crohn disease, ulcerative colitis, pulmonary embolism, deep vein thrombosis, thrombophlebitis, thrombosis of intracranial venous sinus, Budd-Chiari syndrome, and portal vein thrombosis. A logistic regression model was fitted to quantify the increased risk of TE in children with IBD, while adjusting for other risk factors of thrombosis. RESULTS: The total weighted number of pediatric discharges was 7,448,292, and 68,394 (0.92%) were identified with IBD. The incidence of any TE in a hospitalized child or adolescent with IBD was 117.9/10,000 with a relative risk (95% confidence interval) of 2.36 (2.15-2.58). The adjusted odds ratio for any TE in a patient with IBD without surgery was 1.22 (1.08-1.36). Risk factors for TE among patients with IBD include older age, central venous catheter, parenteral nutrition, and an identified hypercoagulable condition. There is an increasing trend of TE in both the IBD and non-IBD patients. CONCLUSIONS: Hospitalized children and adolescents with IBD are at increased risk for TE. Conservative methods of TE prevention including hydration, mobilization, or pneumatic devices should be considered in hospitalized patients with IBD.

Impedance planimetry (EndoFLIP) assisted laparoscopic esophagomyotomy in pediatric population.

INTRODUCTION: Functional lumen imaging probe (EndoFLIP) is a diagnostic technology that assesses esophageal cross-sectional area via impedance planimetry during controlled volumetric distention. The purpose of this study is to evaluate the utility of EndoFLIP intraoperatively during laparoscopic esophagomyotomy. METHODS: We performed a retrospective cohort study reviewing all patients undergoing EndoFLIP assisted laparoscopic esophagomyotomy for achalasia between January and December 2021 (n = 10). Twenty-two patients with achalasia that underwent traditional laparoscopic esophagomyotomy between July 2014 and September 2019 served as a comparison. Primary outcome evaluated was resolution of symptoms at discharge. Secondary outcomes included change in distensibility index (DI), operative time, length of stay, time to regular diet, and reinterventions. RESULTS: All patients managed with EndoFLIP assistance had resolution of dysphagia and postprandial vomiting following intervention. Mean change in DI was 5.32 mm2/mmHg with a myotomy length of 3.6 cm. Operative time was shorter in the EndoFLIP cohort (97 min versus 185 min, p = <0.001). Study patients did not undergo an antireflux operation. There was no difference in length of stay or time to soft diet between groups. All patients were discharged on postoperative day 1 tolerating a mechanical soft diet. No acid suppressive medications were prescribed during the observation period. One patient required dilation for recurrent symptoms and one required reoperation for mucosal leak. CONCLUSION: EndoFLIP assisted laparoscopic esophagomyotomy results in similar short-term outcomes to traditional surgical technique. EndoFLIP allows for focused myotomy length and a shorter operative time. LEVEL OF EVIDENCE: III.

Implementation of enhanced recovery protocols reduces opioid use in pediatric laparoscopic Heller myotomy surgery.

BACKGROUND: Enhanced recovery protocols (ERPs) are effective means of standardizing and improving the quality of surgical care in adults. Our purpose was to retrospectively compare outcomes before and after implementation of ERPs in children undergoing laparoscopic Heller myotomy for achalasia. METHODS: A pediatric-specific ERP was used for children undergoing laparoscopic Heller myotomy starting July 2017 at two pediatric surgery centers within a single metropolitan healthcare system. A retrospective review of 8 patients undergoing Heller myotomies between July 2014 and July 2017 was performed as a control. This cohort was compared to 14 patients managed post-ERP implementation (2017-2020). Outcomes of interest investigated included opioid use during admission, narcotics at discharge, time to regular diet, length of stay (LOS), and readmissions. RESULTS: There was a significant decrease in opioid use both while in the hospital and at time of discharge. Mean morphine equivalent use was 4.50 mg in the pre-ERP cohort and 1.97 mg in the post-ERP cohort. Furthermore, 8 out of 14 (57%) patients in the post-ERP cohort received no opioids during the admission compared with only 2 out of 8 (25%) patients in the pre-ERP cohort. Only 1 out of 14 (7.14%) patients in the post-ERP cohort was discharged with a prescription for opioid medication while 6 out of 8 (75%) in the pre-ERP cohort were discharged with an opiate prescription. CONCLUSIONS: The use of ERP in children undergoing laparoscopic Heller myotomy surgery is safe and effective and leads to a reduction in opioid use during admission and at discharge. LEVELS OF EVIDENCE: Level III.

Colonic hyperactivity results in frequent fecal soiling in a subset of children after surgery for Hirschsprung disease.

BACKGROUND AND AIM: Fecal soiling is a challenging problem in some children after pull-through surgery for Hirschsprung disease (HSCR). The prevailing perception is that soiling results from overflow incontinence; however, its treatment with laxatives yields mixed results. Colonic manometry studies are reported to be normal in most patients in this population. The interpretation of these findings does not support the physiology of fecal overflow incontinence in these children. The aim of the present study was to define the physiology underlying daily, frequent fecal soiling in children after surgery for HSCR using manometric techniques. PATIENTS AND METHODS: Four pediatric motility centers in the United States participated in the study; medical records and manometric tracings (anorectal and colonic) of children (n = 59; 6.5 years; 48 boys) who had pull-through surgery for HSCR and presented with daily, frequent fecal soiling were examined. Children referred for evaluation of constipation who had normal colonic manometry served as controls (n = 25; 6.7 years; 12 boys). The patients with HSCR were divided into 2 groups (Hirschsprung disease groups 1 and 2 [HD1, HD2]) based on the absence or presence of high-amplitude propagated contractions (HAPCs). A control group that included children with chronic constipation was also studied. We compared the mean HAPC frequency between the HD2 and control groups. RESULTS: HD1 included 21 patients who had no HAPCs in fasting or postprandial periods. HD2 included 38 patients who had an average of 0.07 HPACs/min while fasting and 0.13/min in the postprandial state. In this subset the number of HAPCs in the fasting state (P = 0.04) and the postprandial state (P < 0.001) was greater when compared with controls. Additionally, there was a significant increase in HAPCs/min from the fasting to the postprandial state (P = 0.01). In the HD2 group 40% had colonic hyperactivity. CONCLUSIONS: Daily, frequent fecal soiling after pull-through surgery for HSCR may be due to colonic hyperactivity in some children. It is imperative that this unique subset be identified because the management strategy would include avoidance of laxatives, contrary to standard current practice.

Multi-institutional review of bowel management strategies in children with anorectal malformations.

BACKGROUND/RATIONALE: To describe current bowel management program (BMP) strategies in anorectal malformation (ARM) patients based on patient-level predictors using data from a multi-institutional consortium. MATERIALS/METHODS: Patient bowel function and BMP were reviewed from Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) data. The PCPLC is comprised of multidisciplinary specialists researching colorectal and pelvic disorders. Seven US institutions submitted de-identified clinical data on ARM patients into a centralized patient registry. RESULTS: The primary ARM of 624 patients was categorized into Mild (45.2%), Moderate (40.4%) or Complex (14.2%) anomaly classifications. Patient-specific BMP were examined based on age and on the presence of spinal cord/sacral anomalies. 418 (67%) enrolled patients were prescribed BMP (<5 yo 56.4%; ≥5-<12 yo 86.7%; ≥12 81.5%). Constipation was the primary chief complaint (80.2%). Forty percent of patients on a BMP were toilet trained and approximately one-half (48.5%) reported daytime stool accidents. Secondary surgical interventions for antegrade continence enemas (ACE) were examined; 14.5% of patients employed ACE strategies and utilization increased with age and varied based on anatomic anomalies. CONCLUSIONS: This is the first report on BMP strategies for patients with ARM from the Pediatric Colorectal and Pelvic Learning Consortium. Individual patient characteristics are explored for their impact on bowel management strategy utilization. LEVEL OF EVIDENCE: IV.